Neonatal Immune Neutropenia due to Isoantibodies against the Granulocyte Receptor FcγRIIIb.

INTRODUCTION: Neonatal neutropenia is often secondary to sepsis, low birth weight, pregnancy-induced maternal hypertension, and other conditions. CASE REPORT: We report a case of asymptomatic isoimmune neutropenia in a pair of preterm twins. Genotyping confirmed that the mother was negative for HNA-1a, 1b, and 1c, consistent with an FcγRIIIb deficiency. The father was 1(a+b+c-) and the neonates were 1(a-b+c-). A strongly positive result was observed in the granulocyte immunofluorescence test against paternal neutrophils (IgG antibodies). IgG anti-CD16b isoantibodies were detected in the mother's breast milk. Neutropenia resolved after 28 days without requiring any specific treatments. DISCUSSION: Even though neonatal alloimmune neutropenia (NAN) is usually benign and self-limiting, some patients pre-sent with delayed separation of the umbilical cord, mild skin infections, omphalitis, or severe infections like pneumonia, sepsis, and meningitis. Thus, it is important to rule out NAN in case of neonatal neutropenia.

Massive intrasplenic arterial thrombosis in a patient with chronic ITP during the development of an Evans syndrome.

Long-term safety and efficacy of eltrombopag in adults with persitent/chronic primary immune thrombocytopenia (ITP) evaluated in EXTEND study, showed a high response rate (80%) but, in the clinical safety study, it was observed that 6% of the patients presented venous and arterial thrombotic events. In addition, in the course of the disease, autoimmune hemolytic anemia (Evans syndrome, ES) may occur and could increase the risk of thrombosis. We report an interesting case of splenic rupture due to massive intrasplenic arterial thrombosis in the course of ES in a patient with chronic ITP treated with eltrombopag. The purpose of this case report is to highlight the potential increase in thrombotic risk that may involve the use of eltrombopag in hemolysis situations in patients with ITP.

Host immune genetic variations influence the risk of developing acute myeloid leukaemia: results from the NuCLEAR consortium.

The purpose of this study was to conduct a two-stage case control association study including 654 acute myeloid leukaemia (AML) patients and 3477 controls ascertained through the NuCLEAR consortium to evaluate the effect of 27 immune-related single nucleotide polymorphisms (SNPs) on AML risk. In a pooled analysis of cohort studies, we found that carriers of the IL13rs1295686A/A genotype had an increased risk of AML (PCorr = 0.0144) whereas carriers of the VEGFArs25648T allele had a decreased risk of developing the disease (PCorr = 0.00086). In addition, we found an association of the IL8rs2227307 SNP with a decreased risk of developing AML that remained marginally significant after multiple testing (PCorr = 0.072). Functional experiments suggested that the effect of the IL13rs1295686 SNP on AML risk might be explained by its role in regulating IL1Ra secretion that modulates AML blast proliferation. Likewise, the protective effect of the IL8rs2227307 SNP might be mediated by TLR2-mediated immune responses that affect AML blast viability, proliferation and chemorresistance. Despite the potential interest of these results, additional functional studies are still warranted to unravel the mechanisms by which these variants modulate the risk of AML. These findings suggested that IL13, VEGFA and IL8 SNPs play a role in modulating AML risk.